Williams-Campbell syndrome complicated with pulmonary hypertension and Type 2 respiratory failure: An adult case report

Abstract

Williams-Campbell syndrome, is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi, leading to distal airway collapse and bronchiectasis, which typically affects the fourth- to sixth-order bronchi. This article reported a 31-year-old female patient who was diagnosed with Williams-Campbell syndrome with pulmonary hypertension and Type 2 respiratory failure due to extensive cystic bronchiectasis. CT of the thorax showed the affected bronchi had characteristic ballooning on inspiration and collapse on expiration.

Keywords: Williams-Campbell syndrome; bronchiectasis.